| Objective:Autoimmune liver disease(AILD)include primary biliary cholangitis(PBC),autoimmune hepatitis(AIH),primary sclerosing cholangitis(PSC),etc.When patients have two or more autoimmune liver diseases at different stages of the disease,it is called autoimmune liver disease overlap syndrome(OS).Among autoimmune liver disease overlap syndromes,the incidence of primary biliary cholangitis overlap-autoimmune hepatitis overlap syndromes(PBC-AIH overlap syndrome,PBC-AIH OS)is the most common one.Patients with PBC-AIH overlap syndrome have biochemical,immunological and pathological features of PBC and AIH.As a chronic cholestasis disease,the typical pathological manifestation of PBC is obvious bile duct injury,including chronic nonsuppurative destructive cholangitis,bile duct absence,and chronic cholestatic cirrhosis.In fact,the pathological nature of all PBC is intrahepatic bile duct injury.AIH is pathologically characterized with inflammatory infiltration in the liver parenchyma,the pathological essence is hepatocyte injury.At present,it is not clear whether PBC-AIH overlap syndrome effect the liver inflammatory activity,the progression rate and prognosis.This study aimed to investigate the followings:1.The clinical and serological characteristics,as well as the non-invasive liver fibrosis of patients between PBC-AIH overlap syndrome and PBC when the first-seek medical advice;2.Assess the disease activity with biochemical indexes between PBC and PBC-AIH OS patients post 1 year treament;3.Using APRI FIB-4 to assess rate of liver fibrosis progression with PBC-AIH overlap syndrome and PBC patients;4.Evaluate theliver fibrosis progression betaeen PBC and PBC-AIH OS patients with APRI and FIB-4.Methods:We collected the clinical data of 79 patients with PBC-AIH overlap syndrome(PBC-AIH OS group)and 97 patients with PBC(PBC group)who were admitted to the Second Affiliated Hospital of Kunming Medical University from January 2013 to December 2019.Then compared and analyzed Clinical characteristics,laboratory tests,and non-invasive liver fibrosis indicators FIB-4 and APRI in each group on admission.After that,Clinical characteristics,laboratory tests,and non-invasive liver fibrosis indicators FIB-4 and APRI in each group on admission were analyzed.We also calculated APRI and FIB-4 indexes in each group after 1 year treatment and the last follow up.Biochemical response in each group was assessed with Barcelona criteria.The incidence of cirrhosis and adverse events in each group was compared during follow-up.Results:1.79 patients(79/176;44.9%)in the PBC-AIH OS group were included in this study,including 6 males and 73 females,with an average age of 57.43±9.44 years.There were 97 patients in the PBC group,including 8 males and 89 females,with an average age of 58.30±10.45 years.The clinical manifestations of the two groups of patients upon admission were as follows:fatigue,jaundice,gastrointestinal bleeding and abdominal pain.At least 25%of the patients were asymptomatic and presented to the doctor due to abnormal liver function;2.There were no significant differences in gender,age,symptoms,Child classification,BMI,UDCA treatment amount,pathological staging,and immunological indicators(AMA-M2,GP210,SP100)between patients of PBC-AIH OS group and PBC group;3.There was no statistical difference between PBC-AIH OS group and PBC group with extrahepatic autoimmune diseases(P>0.05);there was no statistical difference between patients of PBC-AIH OS group and PBC group couple with hypertension,diabetes,and myocardial/cerebral infarction,(P>0.05);4.The ALT,AST,TCHO,Hb,and PLT in PBC-AIH OS group were significantly higher than those in PBC group,the difference was statistically significant,P<0.05.Cr and BUN in the PBC-AIH OS group were significantly lower than those in the PBC group,the difference was statistically significant(P<0.05).There was no significant difference between PBC-AIH OS group and PBC group in ALP,GGT,ALB,TG,TBil,Dbil,PT,APTT,RBC,WBC,IgA,IgG,IgM in first visit to hospital(P>0.05);5.No statisticall difference were observed in APRI and FIB-4,APRI and FIB-4 grades between patients of PBC-AIH OS group and PBC group were in first visit to hospital;6.The serum ALP,GGT,ALT and AST levels of the patients of PBC-AIH OS group and PBC group after UDCA treatment for 1 year were significantly lower than pretreatment.There was no significant difference in ALP,GGT,ALT and AST between patients of PBC-AIH OS group and PBC group after 1 year of UDCA treatment.7.APRI,FIB-4,APRI grades,FIB-4 grades,APRI changes,FIB-4 changes,APRI and FIB-4 grades changes were not statistically different between patients of PBC-AIH OS group and PBC group after 1 year of treatment;8.No statistical difference were found in APRI,FIB-4,APRI grades,FIB-4 grade,APRI change,FIB-4 change,APRI annual change,FIB-4 annual change,APRI grade change,FIB-4 grade change,between patients of PBC-AIH OS group and PBC group at the last follow-up;9.There was no statistical difference in the biochemical remission rate between patients of PBC-AIH OS group and PBC group after 1 year treatment and at the last follow-up;10.There was no statistical difference in the occurrence of liver cirrhosis and liver disease-related adverse events during the follow-up period between patients of PBC-AIH OS group and PBC group.Conclusion:1.In our long-term follow-up,44.9%of PBC patients had PBC-AIH overlap syndrome,and 31%of PBC patients had a tendency to develop PBC-AIH overlap syndrome.PBC patients with elevated serum ALT and AST are more likely to suffer from PBC-AIH overlap syndrome.At this point,all PBC patients need to be screened for PBC-AIH overlap syndrome;2.There was no difference in the efficacy of UDCA between patients with PBC-AIH overlap syndrome and patients with PBC;3.After standard treatment,there was no difference in the degree of disease activity and the rate of liver fibrosis progression between PBC-AIH overlap syndrome and PBC patients;4.There was no difference in 3 year of the short-term prognosis between PBC-AIH and PBC patients. |
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